Update on the COVID-19-associated inflammatory syndrome in children and adolescents; paediatric inflammatory multisystem syndrome-temporally associated with SARS-CoV-2.

We provide an update on the state of play with regards a newly described inflammatory condition which has arisen during the current SARS-CoV-2 pandemic. The condition has been named paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 or multisystem inflammatory syndrome in children. This condition has shown significant similarities to Kawasaki disease and toxic shock syndrome.

Periodic fevers and autoinflammatory syndromes in childhood.

Recurrent fever is a common presentation in paediatric practice and can be caused by a wide variety of diseases including autoinflammatory conditions. The innate immune system plays an essential role in the 'first line' response to infection through mediation of inflammatory responses. Inflammasomes are part of the regulatory process for this system and result in the production of the powerful pro-inflammatory cytokine interleukin-1B. Dysregulation of inflammasomes, and Interleukin 1 production, contributes to the pathogenesis of autoinflammatory diseases. This review focuses on described periodic fever syndromes (PFS) which are now collectively referred to as autoinflammatory syndromes. Conditions discussed include periodic fever aphthous stomatitis pharyngitis and cervical adenopathy, familial Mediterranean fever, tumour necrosis factor receptor-associated periodic syndromes, hyperimmunoglobulinaemia D and the cryopyrin-associated periodic syndromes. Presenting features, complications, diagnostic and treatment approaches for these conditions are discussed. Nonetheless, as most of these conditions are rare and may have significant long-term complications, it is recommended that they be managed in consultations with a physician experienced in managing PFS.

Juvenile idiopathic arthritis in the new world of biologics.

Juvenile idiopathic arthritis results in significant pain and disability in both children and adults. Advances in treatment resulting in improved long-term outcomes have occurred; however, an emphasis on early and aggressive diagnosis and management hopes to improve outcomes further. Juvenile idiopathic arthritis remains a clinical diagnosis of exclusion, but further research may delineate biological markers associated with the disease and its subtypes. Therapy for patients includes intra-articular steroid injections, disease modifying agents such as methotrexate and biological agents. Biological agents have provided exciting new therapeutic options in the last decade; however, long-term side effects of modulating the immune system are not yet fully understood. Systemic steroids may also be required but their long-term use is avoided. Uveitis needs to be screened for in all of those with the diagnosis. Multidisciplinary team care is required in managing these young people.

A feasibility study of the effect of intra-articular corticosteroid injection on isokinetic muscle strength in children with juvenile idiopathic arthritis.

This study assessed the magnitude of changes in isokinetic muscle strength in children with juvenile idiopathic arthritis (JIA) before and after treatment with intra-articular corticosteroid injection and assessed the feasibility of a larger study of the same effect. Isokinetic dynamometry was used to measure peak knee extension and flexion torque in 12 children before and after treatment for unilateral knee arthritis. Extensor and flexor strength was reduced on the affected side before treatment (-0.56 Nm/kg, p = .004 and -0.24 Nm/kg, p = .02 respectively). Increases in extensor strength were observed at two weeks (p = .01) and twelve weeks postinjection (p = .03). Improvements at 6 weeks approached but did not reach statistical significance (p = .17). Improvements in flexor strength were not observed until 12 weeks postinjection (p = .03). Despite significant improvements in extensor strength, low peak knee extensor torque continued to be observed at 12 weeks (p = .01). Knee extensor and flexor strength is reduced in children with JIA with active arthritis and improves following intra-articular corticosteroid injection. Significant improvements in knee extensor and flexor strength were seen postinjection; however deficits in extensor strength were still evident at three months. Isokinetic dynamometry was safe and well tolerated in our sample of children with JIA with active arthritis.

Multisystem Inflammatory Syndrome in New Zealand Children.

New Zealand (NZ) initially adopted an elimination approach to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Pre-Omicron variant, the NZ pediatric population was immunologically naïve to SARS-CoV-2. This study, utilizing national data sources, describes the NZ incidence of multisystem inflammatory syndrome in children (MIS-C) following infection with the Omicron variant. MIS-C incidence was 1.03 of 100,000 age-specific population and 0.04 of 1000 recorded SARS-CoV-2 infections.

Incidence, Clinical Manifestations, and Severity of Juvenile Idiopathic Arthritis Among Maori and Pacific Island Children.

OBJECTIVE: To describe the incidence, demographics, diagnostic clinical manifestations, and severity of juvenile idiopathic arthritis (JIA) in Maori and Pacific Island children compared to European children. METHODS: A chart review was conducted of all children with JIA seen by Auckland pediatric and rheumatology services between the years 2000 and 2015. Demographic data and diagnostic clinical manifestations, including poor prognostic features, were collated. The incidence, diagnostic, clinical manifestations, and severity of JIA were determined and compared between ethnic groups, in particular Maori, Pacific Island, and European children. RESULTS: The overall incidence in a New Zealand cohort of children with JIA was 5.1/100,000 children per year, which was significantly higher among European children (7.2/100,000 children per year) compared to all other ethnic groups. Poor prognostic features at diagnosis were present in 36% of children with JIA, with significantly more Maori and Pacific Island children presenting with poor prognostic features compared to European children (58% versus 27%; P = 0.0001). Maori and Pacific Island children had significantly more poor prognostic features per child associated with JIA (1.10 versus 0.37; P < 0.0001) and in oligoarticular and polyarticular JIA (1.28 versus 0.40; P < 0.0001), which was independent of socioeconomic status. Significant features included cervical involvement (25% versus 9%; P = 0.03), erosive changes (22% versus 8%; P = 0.05), joint space narrowing (13% versus 2%; P = 0.02), and positive rheumatoid factor polyarticular disease (47% versus 14%; P = 0.01). CONCLUSION: Maori and Pacific Island children were more likely to present with poor prognostic features at diagnosis, although the incidence of JIA was demonstrated to be significantly higher among European children compared to all ethnic groups.

Paediatric non-IgE mediated food allergy: guide for practitioners.

AIM: Food avoidance in children is increasingly common due to concerns about allergy. We aim to review the current literature on paediatric non-IgE mediated food allergy including what is known about pathophysiology, diagnosis, management and prognosis of common and severe presentations. Considerations regarding appropriate formula selection are also presented. METHODS: Common non-IgE mediated conditions were searched through common medical databases. Thorough review of available literature was then synthesised and critically appraised. RESULTS: Current understanding of immunological mechanisms of most non-IgE mediated conditions remains elusive. Most conditions are outgrown in childhood and have a good prognosis. Dietary modification for some conditions is important to ensure safety. They are not recommended in all situations due to potentially harmful consequences. CONCLUSION: Assessment of children with concerns regarding non-IgE mediated conditions requires a thorough history and is generally not supported by reliable diagnostic tests. Caution is warranted when advising families to undertake dietary exclusions unless well supported by the evidence and ensuring benefits outweigh any potential harm.

Maternal stress associated with juvenile idiopathic arthritis.

OBJECTIVE: To assess parental stress levels of mothers of children with juvenile idiopathic arthritis (JIA) aged between 2-12 years and compare with those reported for other chronic childhood illnesses. METHODOLOGY: Mothers of children aged between 2-12 years with JIA were recruited from hospital-based outpatient clinics. Maternal stress was measured by using the Parenting Stress Index Long Form (PSI). The physician assessing the child completed an active joint count, a physician's global assessment and recorded the C-reactive protein and/or erythrocyte sedimentation rate if one was clinically indicated. RESULTS: The mothers recruited had children with a mean age of 6 years. The mean total stress score of mothers of children with JIA measured by the PSI was 235.4 (95% CI 218.5-252.3) was greater than the mean total stress scores for mothers of normal children at 222.8 (95% CI 221.4-224.2). It was also greater than children with other chronic disorders such as insulin-dependent diabetes mellitus (IDDM), 218.1 (95% CI 204.7-231.6) and profound deafness, 221.7 (95% CI 206.4-237.0). One third of mothers had total PSI scores that were in the clinical range (Total PSI > 260), indicating a need for intervention. CONCLUSION: JIA should be regarded as a significant illness in which maternal stress is at least equivalent to that associated with the care of children with other chronic diseases of childhood.